title: Long-term outcome of bilateral Wilms tumors (BWT).
abstract: BACKGROUND
  Modern WT management consist of ample chemotherapy, nephron-sparing surgery, and, when indicated, radiotherapy. Survivors may develop renal failure or secondary tumors due to anticancer treatment. We analyzed long-term outcome (follow-up >5 years) after bilateral Wilms tumor (BWT) treatment with respect to survival, renal function, and secondary malignancies.
  
  
  METHODS
  From 41 patients (23 females, 28 synchronous tumors) diagnosed with BWT between 1967 and 2007, 25 (18 females, 14 synchronous) with a follow-up >5 years could be included. Of this subgroup, median age at diagnosis was 1.64 years (range 0.27-5.35), and at maximum follow-up 14.99 years (range 5.40-33.99). Data were retrospectively collected and analyzed.
  
  
  RESULTS
  One patient (4%) died 17.75 years after diagnosis, five (20%) had renal transplants: 3/5 after bilateral nephrectomy for Denys-Drash syndrome (DDS), and 2/5 for ESRD after an interval of 7 and 18 years, respectively. All transplanted patients remained in CR. Another three patients developed mild renal insufficiency (creatinine levels 1.3, 1.8, and 2.8 mg/100 ml, respectively; N = 0.5-1.2), combined with hypertension in 1; neither of them was transplanted. Sixteen (64%) had normal renal function and were in CR. Long-term renal function appeared significantly better after bilateral nephron sparing surgery (NSS) then after other surgical procedures (P < 0.0001). Seven secondary tumors were found in five (20%) patients, one of whom had a DDS.
  
  
  CONCLUSIONS
  Long-term 10-year overall survival was 78%. There was significant morbidity (13/25, 52%), in terms of renal failure (8/25, 32%) including renal transplantation (5/25, 20%), and secondary tumors (5/25). These findings necessitate long-term follow-up beyond childhood. Future work should be directed at reducing the harmful effects of treatment, including the increased use of NSS.
authors.affiliation: Pediatric Surgical Center of Amsterdam (ECH-AMC/VUmc), Academic Medical Center, University of Amsterdam, Amsterdam. d.aronson@chir.umcn.nl
authors.forename: Daniel C
authors.initials: DC
authors.lastname: Aronson
authors.forename: Annelie
authors.initials: A
authors.lastname: Slaar
authors.forename: Richard C
authors.initials: RC
authors.lastname: Heinen
authors.forename: Jan
authors.initials: J
authors.lastname: de Kraker
authors.forename: Hugo A
authors.initials: HA
authors.lastname: Heij
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007677:Kidney Function Tests; D007680:Kidney Neoplasms / Q000628:therapy*; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000628:therapy*; D009392:Nephrectomy; D011879:Radiotherapy Dosage; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D009396:Wilms Tumor / Q000628:therapy*; D055815:Young Adult